Still Here...



So, I was supposed to be writing this update from HOME.... but Eithene had other ideas, sigh.

We have a date for her manometry testing and intestinal biopsies- July 15th. Since she had been fever-free for about 30 hours, and is on a good IV antibiotic, we decided to go home and come back on July 13th for the next round of testing.

 We spent all day yesterday arranging TPN deliveries etc, and none of us really paid attention to the fact that even back on full-time O2 Eithene's heart rate was really high all day. (which, as a side-note: the tachycardia is thought to be autonomic, so she's just staying on O2 until her body flips back out of it...). 

 Unfortunately, it seems that we should have been a little more observant as when we checked her temp that evening it was about 101F (high for her). This meant re-sending blood, urine and stool cultures, and getting chest x-rays etc. This morning we were still talking about going home, but the infection she has right now (pseudomonas) is only sensitive to 2 IV abx, and they are limited spectrum. THis means any other infections will likely need a different IV abx, and due to special issues specific to Eithene and her TPN needs (as well as the differences btwn hospital and home capabilities) we would not be able to run more than one IV abx at home. So, we would have to come straight back inpatient for ANY infections.....

 Therefore,we decided to stay until tomorrow morning to get the cultures back- and then hopefully go home.

 Unfortunately, I'm  not anticipating a quick discharge anymore, as Eithene suddenly started to look and act pretty sick a few hours after waking up. She is having huge sweats again- even on the tylenol- looking very pale, and feeling badly. So, I hope the urine grows something easily treatable. At this point I'm worried that if we can't catch whatever this is in time, we won't be able to get our GI testing done on July 16th. We have to be off of all antibiotics for at least several days before manometry testing as the abx completely change her motility and will make the testing totally inaccurate. Its a fine balance- because is we keep her off the meds too long, she will get another infection and then we won't be able to do the testing at all!

 On the endocrine front, we completed all the testing and didn't get as much information as we had hoped for. Eithene has an abnormal, but still adequate, response to both glucagon and ACTH (stimulates the adrenal gland). As such, we can say that primary adrenal insufficiency and glycogen storage diseases are both NOT the cause of the hypoglycemia.

This means the problem is in her pancreas- which is what we had anticipated. Eithene has exocrine pancreatic defects (blood sugar issues are endocrine defects) as well as vast GI issues. The pancreatic tissue and GI tract develop at the same time from the same embryonic tissue, so the Endocrinologist thinks its likely all the systems that developed from this affected tissue have problems. Unfortunately, the medication he had hoped to trial on Eithene is FAR too risky. When he first mentioned it he wasn't aware of her other looooooong list of medical issues. So, this leaves us with limited options. There is another medication we can use, but it also has many side-effects and can be hard on the kidneys... so we would like to avoid it if possible. We know that the TPN is definitely making Eithene's hypoglycemia worse (the constant sugar infusion further encourages her insulin regulation to become abnormal), so we think our best option is a complicated TPN wean down from 24hrs to a goal of 20 hours. It will take about 1 month to accomplish this, if it works. I am hopeful we can get to at least 22 hrs on with 2 off- but we are not even going to attempt it until after the GI testing.

Right now we are just resting and hoping to get to the bottom of this current illness as quickly as possible.